RESUMO
Granulomatosis with polyangiitis is a systemic vasculitis. While the classic triad typically comprises otorhinolaryngologic, pulmonary, and renal manifestations, it is essential to recognize that granulomatosis with polyangiitis can affect any organ. Furthermore, reports have documented less common sites of involvement, such as the gastrointestinal tract. In this case-based review, we focus on a case of granulomatosis with polyangiitis presenting with intestinal perforation and the added challenge of concurrent pancytopenia.A 25-year-old female was diagnosed with granulomatosis with polyangiitis, with her clinical course progressing from joint pain to severe multi-organ involvement, including gastrointestinal complications. Treatment challenges emerged with the development of pancytopenia. While this may not directly result from granulomatosis with polyangiitis, it introduced an additional layer of complexity and delayed the induction of remission with immunosuppressants. Despite initial stabilization, an unexpected jejunal perforation occurred, requiring surgical intervention and subsequent postoperative care. The case underscores the complex nature of granulomatosis with polyangiitis and its potential complications. A literature search yielded discrete relevant cases in the context of our patient's intricate presentation, which has been summarized.We highlight the complexities in diagnosing and managing granulomatosis with polyangiitis-related complications, especially in uncommon presentations, and emphasize the importance of a personalized approach to patient care in these circumstances.
Assuntos
Granulomatose com Poliangiite , Perfuração Intestinal , Pancitopenia , Humanos , Granulomatose com Poliangiite/complicações , Granulomatose com Poliangiite/tratamento farmacológico , Granulomatose com Poliangiite/diagnóstico , Feminino , Perfuração Intestinal/etiologia , Perfuração Intestinal/cirurgia , Adulto , Pancitopenia/etiologia , Pancitopenia/terapia , Imunossupressores/uso terapêutico , Resultado do Tratamento , Doenças do Jejuno/etiologiaRESUMO
OBJECTIVES: To investigate the effectiveness of donor-derived chimeric antigen receptor T (CAR-T) cells in the treatment of relapsed cases after allogeneic hematopoietic stem cell transplantation (allo-HSCT), and whether donor-derived peripheral blood stem cells (PBSCs) have a therapeutic effect on pancytopenia after CAR-T cell therapy. METHODS: We analyzed data from five adults with B-cell acute lymphoblastic leukemia (ALL) who had relapse after allo-HSCT and received donor-derived CAR-T cell therapy and donor-derived PBSCs to promote hematopoietic recovery. RESULTS: All patients had negative minimal residual disease after CAR-T therapy, grade 1-2 cytokine release syndrome, and developed grade 4 hematologic toxicity. During the pancytopenia stage after CAR-T cell therapy, donor-derived PBSCs were transfused without graft-versus-host disease (GVHD) prophylaxis. Four patients had grade I-II acute GVHD (aGVHD). After corticosteroid treatment, aGVHD resolved and hematopoiesis was restored. Although steroids in combination with etanercept and ruxolitinib relieved symptoms in one patient with grade IV aGVHD, complete hematopoietic recovery was not achieved, and the patient died due to severe infection. CONCLUSIONS: Donor-derived CAR-T cell therapy is safe and effective in patients with relapsed/refractory ALL after allo-HSCT. Donor-derived PBSCs infusion could achieve hematopoietic recovery with controllable aGVHD in patients with persistent pancytopenia.
Assuntos
Doença Enxerto-Hospedeiro , Transplante de Células-Tronco Hematopoéticas , Pancitopenia , Receptores de Antígenos Quiméricos , Humanos , Antígenos CD19 , Doença Enxerto-Hospedeiro/diagnóstico , Doença Enxerto-Hospedeiro/etiologia , Doença Enxerto-Hospedeiro/terapia , Transplante de Células-Tronco Hematopoéticas/efeitos adversos , Imunoterapia Adotiva/efeitos adversos , Pancitopenia/diagnóstico , Pancitopenia/etiologia , Pancitopenia/terapia , Linfócitos TRESUMO
BACKGROUND: Mutations in the DNA ligase IV (LIG4) gene cause a rare autosomal recessive disorder called LIG4 deficiency syndrome. The LIG4 deficiency is featured by severe disorders, including combined immunodeficiency disease, special face ("bird-head-like" face), developmental delays, pancytopenia, and radiosensitivity. Currently there are no curative treatment options except potentially by performing a hematopoietic stem cell transplantation (HSCT). CASE PRESENTATION: Here we reported the clinical course of a 4 and 1/2-year-old Chinese female with LIG4-deficiency featured with pancytopenia, severe growth retardation (weight of 13.5 kg, < 3rd percentile), length of 100 cm (<2d percentile), head circumference of 46 cm (<3rd percentile), and mild microcephaly. Despite regular IVIG administrations (5 g, once a month), the patient's thrombocytopenia had progressed. Eventually, the patient received HSCT that successfully normalized the LIG4 syndrome associated pancytopenia and corrected the LIG4 mutation. Despite progress the patient succumbed to thrombotic microangiopathy more than 3 months after HSCT. CONCLUSIONS: This case reports an example of partially successful HSCT as a treatment option for LIG4 syndrome. It is possible that individual factors influence the therapeutic effect of HSCT in LIG4 deficiency.
Assuntos
Transplante de Células-Tronco Hematopoéticas , Síndromes de Imunodeficiência , Pancitopenia , Feminino , Humanos , Pancitopenia/terapia , Síndromes de Imunodeficiência/genética , Transtornos do Crescimento/genéticaRESUMO
Acute viral hepatitis, including hepatitis A, B, E, D, and G, can lead to severe bone marrow suppression due to cytotoxic lymphocytes. The bone marrow suppression causes aplastic anaemia which is mostly unresponsive to immunosuppressive therapy. Such patients require bone marrow transplant for a complete cure. The pancytopenia can evolve during recovery from transaminitis. We are presenting two case reports relating aplastic anaemia with acute viral hepatitis in two young patients-23 and 16 years of age. The 23-year-old female patient had hepatitis A associated with aplastic anaemia whereas the young 16-year-old male patient was diagnosed with Hepatitis E IgG associated aplastic anaemia. Unfortunately, the first patient could not cope with the complications relating to pancytopenia and was unable to reach the bone marrow transplant stage. The second patient did not have a bone marrow transplant but showed an excellent response to immunosuppressive therapy before the transplant and survived.
Assuntos
Anemia Aplástica , Hepatite A , Hepatite E , Pancitopenia , Feminino , Masculino , Humanos , Adulto Jovem , Adulto , Adolescente , Anemia Aplástica/complicações , Anemia Aplástica/terapia , Pancitopenia/etiologia , Pancitopenia/terapia , Transplante de Medula ÓsseaRESUMO
Autoimmune pancytopenia is rarely seen with Hodgkin lymphoma, and only one pediatric case of pancytopenia after autologous hematopoietic stem cell transplantation (HSCT) has been reported. We herein report a case of autoimmune pancytopenia that developed after autologous HSCT for nodular lymphocyte predominant Hodgkin lymphoma (NLPHL). A 56-year-old Japanese woman underwent autologous HSCT for NLPHL. She developed autoimmune pancytopenia seven months after autologous HSCT. In this case, PSL was effective, and the blood cell counts normalized completely. However, the patient suffered from a fatal infection, probably because of immunosuppression caused by prolonged administration of PSL, as well as a history of several chemotherapies and autologous HSCT. To our knowledge, this is the first adult case of autoimmune pancytopenia after autologous HSCT for Hodgkin lymphoma. To further validate the optimal treatment strategy for autoimmune cytopenia after autologous HSCT, more cases are necessary.
Assuntos
Transplante de Células-Tronco Hematopoéticas , Doença de Hodgkin , Pancitopenia , Adulto , Feminino , Humanos , Criança , Pessoa de Meia-Idade , Doença de Hodgkin/terapia , Doença de Hodgkin/patologia , Pancitopenia/etiologia , Pancitopenia/terapia , Recidiva Local de Neoplasia , Transplante de Células-Tronco Hematopoéticas/efeitos adversos , Transplante AutólogoRESUMO
The patient was a 78-year-old man. After 4 courses of GEM plus nab-PTX therapy for multiple recurrent liver metastases after pancreatic body cancer surgery, the patient was aware of general malaise and edema of the extremities. Blood tests showed pancytopenia, and he was admitted to the hospital with a diagnosis of chemotherapy-induced pancytopenia. On the second day, hemolytic anemia with crushed red blood cells was observed, suggesting thrombotic microangiopathy (TMA). Considering the possibility of thrombotic thrombocytopenic purpura(TTP), the patient was started on plasma exchange with steroids. After 7 days of plasma exchange, his thrombocytopenia, hemolytic anemia, and renal dysfunction improved, and he was discharged from the hospital on the 28th day. Although GEM-induced TMA is a life-threatening complication, there is no established treatment for it. We report a case of GEM-induced TMA that was successfully treated with plasma exchange.
Assuntos
Anemia Hemolítica , Neoplasias Pancreáticas , Pancitopenia , Púrpura Trombocitopênica Trombótica , Microangiopatias Trombóticas , Masculino , Humanos , Idoso , Troca Plasmática/efeitos adversos , Pancitopenia/terapia , Microangiopatias Trombóticas/induzido quimicamente , Microangiopatias Trombóticas/terapia , Púrpura Trombocitopênica Trombótica/induzido quimicamente , Púrpura Trombocitopênica Trombótica/diagnóstico , Púrpura Trombocitopênica Trombótica/terapia , Anemia Hemolítica/etiologia , Neoplasias Pancreáticas/tratamento farmacológico , Neoplasias Pancreáticas/complicações , Neoplasias PancreáticasRESUMO
Rapid advances in sequencing technology have led to the identification of somatic mutations that predispose a significant subset of the aging population to myeloid malignancies. Recently recognized myeloid precursor conditions include clonal hematopoiesis of indeterminate potential (CHIP) and clonal cytopenia of unknown significance (CCUS). These conditions can present diagnostic challenges and produce unwarranted anxiety in some instances. While the risk of progression to myeloid malignancies is very low in CHIP, true CCUS confers an exponential increase in risk. Idiopathic cytopenia of unknown significance (IDUS) lacks the predisposing genetic mutations and has a variable course. In this review we define the early myeloid precursor conditions and their risk of progression. We present our diagnostic approach to patients with unexplained cytopenias and discuss the clinical consequences of CHIP and CCUS.
Assuntos
Hematopoiese Clonal , Pancitopenia/genética , Idoso de 80 Anos ou mais , Gerenciamento Clínico , Hematopoese , Sequenciamento de Nucleotídeos em Larga Escala , Humanos , Masculino , Mutação , Pancitopenia/diagnóstico , Pancitopenia/terapiaRESUMO
INTRODUCTION: Autoimmune hemolytic anemia (AIHA), immune thrombocytopenia (ITP), and autoimmune neutropenia (AIN) are reported in the literature after liver, intestinal, heart, pancreas, and kidney transplants. We report a case of autoimmune pancytopenia (AIHA, AIN and ITP) 9 years after liver transplantation with confirmed erythrocyte and neutrophil auto-antibodies. CASE REPORT: A 49 years old man was admitted to our hospital presented with dysentery and fever, with history of liver transplantation in 2008. Laboratory evaluation demonstrated hemoglobin: 7.2â¯g/dL, granulocytes: 0.10â¯×â¯109/L and platelets: 15â¯×â¯109/mm³; indirect bilirubin: 3.62â¯mg/dL; lactate dehydrogenase: 603 U/L. Direct antiglobulin test revealed a monospecific anti-IgG plus C3 and the acid eluate was reactive to all panel red cells, consistent with an AIHA. Granulocyte immunofluorescence test (GIFT) and agglutination test (GAT) were reactive for granulocytes. Test with Luminex technology for human neutrophil antigen (HNA) antibody detection was strong reactive with beads expressing HNA-1a, -1b, -1c, -2, -4a and -5a antigens. HNA genotyping revealed the presence of the corresponding antigens, confirming the autoantibodies. Test with Luminex technology for human leucocyte antigen (HLA) antibody detection was negative. Monoclonal antibody immobilization of platelet antigens (MAIPA) assay was negative. Viral causes were excluded. The condition was compatible with clinical onset of autoimmune pancytopenia. Prednisone was administered at an initial dose of 1â¯mg/kg/day and immunosuppressive therapy was adjusted. This treatment resulted in rapid resolution of pancytopenia. CONCLUSION: Combined autoimmune pancytopenia (AIHA, AIN and ITP) is a rare condition that may occur after liver transplantation. Early recognition of this phenomenon permits appropriate treatment.
Assuntos
Autoanticorpos/sangue , Doenças Autoimunes , Terapia de Imunossupressão , Transplante de Fígado , Pancitopenia , Prednisolona/administração & dosagem , Doenças Autoimunes/sangue , Doenças Autoimunes/etiologia , Doenças Autoimunes/terapia , Humanos , Masculino , Pessoa de Meia-Idade , Pancitopenia/sangue , Pancitopenia/etiologia , Pancitopenia/terapiaRESUMO
The COVID-19 pandemic has affected the health and healthcare of individuals of all ages worldwide. There have been multiple reports and reviews documenting a milder effect and decreased morbidity and mortality in the pediatric population, but there have only been a small number of reports discussing the SARS-CoV-2 infection in the setting of an inborn error of metabolism (IEM). Here, we report two patients with underlying metabolic disorders, propionic acidemia and glutaric aciduria type 1, and discuss their clinical presentation, as well as their infectious and metabolic management. Our report demonstrates that individuals with an underlying IEM are at risk of metabolic decompensation in the setting of a COVID-19 infection. The SARS-CoV-2 virus does not appear to cause a more severe metabolic deterioration than is typical.
Assuntos
Erros Inatos do Metabolismo dos Aminoácidos/complicações , Encefalopatias Metabólicas/complicações , COVID-19/complicações , Glutaril-CoA Desidrogenase/deficiência , Acidemia Propiônica/complicações , SARS-CoV-2 , Acidose/etiologia , Acidose/terapia , Acidose Láctica/etiologia , Transfusão de Componentes Sanguíneos , COVID-19/diagnóstico , Teste de Ácido Nucleico para COVID-19 , Terapia Combinada , Proteínas Alimentares/administração & dosagem , Gerenciamento Clínico , Suscetibilidade a Doenças , Ingestão de Energia , Nutrição Enteral , Feminino , Hidratação , Glucose/administração & dosagem , Glucose/efeitos adversos , Humanos , Hiperamonemia/etiologia , Hiperamonemia/terapia , Hiperglicemia/induzido quimicamente , Hiperglicemia/tratamento farmacológico , Lactente , Insulina/uso terapêutico , Unidades de Terapia Intensiva Pediátrica , Oxigenoterapia , Pancitopenia/etiologia , Pancitopenia/terapia , Diálise Renal , Síndrome de Resposta Inflamatória Sistêmica/diagnósticoRESUMO
As the Severe Acute Respiratory Syndrome Coronavirus 2 (SARS-CoV2) pandemic progresses, various hematologic complications have emerged, often centered around the hypercoagulable state. However, pancytopenia represents a rare but serious complication from SARS-CoV2 infection. While lymphopenia is a common finding, concomitant acute anemia and thrombocytopenia are not commonly reported. We describe a novel case of SARS-CoV2 pancytopenia in a 40-year-old male without active risk factors for cell line derangements but subsequent critical illness.
Assuntos
Injúria Renal Aguda/etiologia , COVID-19/complicações , Pancitopenia/virologia , Insuficiência Respiratória/etiologia , SARS-CoV-2/patogenicidade , Injúria Renal Aguda/terapia , Adulto , COVID-19/diagnóstico , Terapia de Substituição Renal Contínua , Humanos , Masculino , Pancitopenia/terapia , Respiração Artificial , Insuficiência Respiratória/terapiaRESUMO
INTRODUCTION: Ectopic pregnancy (EP) is an emergency condition in the gynecologic field. Methotrexate (MTX) is a drug of choice for the medical treatment of EP. Severe adverse events are rare among patients treated with MTX for this condition. REASON FOR REPORT: We describe a woman with severe multi-organ involvement experiencing about six days of instability after treatment with just a single-dose MTX for EP. This life-threatening condition is not common with a single dose of MTX. A 30-year-old healthy woman was treated medically with MTX for an EP. Three days later the patient was admitted to the emergency department of our hospital with generalized pustular rashes, alopecia, hyperpigmentation, nausea and vomiting, oral ulcers, and raised Creatinine level. Four days later due to pancytopenia, fever, and loss of consciousness, she was transferred to the intensive care unit and was intubated. OUTCOME: After 38 days of hospitalization, treatment was successful with leucovorin and supportive care and the patient's symptoms and clinical manifestations were regressed.
Assuntos
Abortivos não Esteroides/efeitos adversos , Hipersensibilidade a Drogas/etiologia , Metotrexato/efeitos adversos , Adulto , Alopecia/induzido quimicamente , Alopecia/terapia , Antibacterianos/uso terapêutico , Hipersensibilidade a Drogas/terapia , Eritropoetina/uso terapêutico , Feminino , Febre/induzido quimicamente , Febre/terapia , Fator Estimulador de Colônias de Granulócitos/uso terapêutico , Humanos , Hiperpigmentação/induzido quimicamente , Hiperpigmentação/terapia , Meropeném/uso terapêutico , Pancitopenia/induzido quimicamente , Pancitopenia/terapia , Transfusão de Plaquetas , Gravidez , Gravidez Ectópica/tratamento farmacológico , Infecções por Pseudomonas/tratamento farmacológico , Pseudomonas aeruginosa , Inconsciência/induzido quimicamente , Inconsciência/terapiaAssuntos
COVID-19/complicações , Pancitopenia/etiologia , Hemorragia Retiniana/etiologia , Adulto , Transfusão de Componentes Sanguíneos , COVID-19/patologia , COVID-19/terapia , Humanos , Terapia a Laser , Masculino , Pancitopenia/patologia , Pancitopenia/terapia , Hemorragia Retiniana/patologia , Hemorragia Retiniana/terapia , SARS-CoV-2/isolamento & purificação , Resultado do TratamentoRESUMO
OBJECTIVE: Mutations in IKZF1, which encodes Ikaros family zinc finger 1 (IKAROS) transcription factor, are associated with recurrent infections, cytopenia, autoimmune diseases, and hematologic malignancies. Diverse clinical phenotypes resulting from IKZF1 mutations include pulmonary fungal infections, cytopenia, autoimmune hemolytic anemia (AIHA), and malignancies. In this study, we aimed to assess the DNA-binding ability and pericentromeric (PC) localization of a variant of IKZF discovered in a patient. MATERIALS AND METHODS: DNA-binding ability of a pathogenic IKZF variant was tested using electrophoretic mobility shift assay and PC localization of the variant was assessed by immunofluorescent microscopy in NIH3T3 cells. RESULTS: Clinical features of a 3-month-old male infant who underwent hematopoietic stem cell transplantation because of an IKZF1 mutation-associated common variable immunodeficiency, AIHA, and pancytopenia are described. DNA studies revealed a heterozygous missense variant (IKZF1 NM_006060 c.427C>T; p.R143W). Cotransfection studies revealed that mutant R143W has a partial dominant-negative effect over PC targeting and DNA binding. CONCLUSIONS: IKZF1 mutation must be kept in mind if neonatal AIHA, common variable immunodeficiency, and pancytopenia are observed.
Assuntos
Anemia Hemolítica Autoimune/genética , Imunodeficiência de Variável Comum/genética , Fator de Transcrição Ikaros/genética , Pancitopenia/genética , Anemia Hemolítica Autoimune/complicações , Anemia Hemolítica Autoimune/terapia , Animais , Imunodeficiência de Variável Comum/complicações , Imunodeficiência de Variável Comum/terapia , Transplante de Células-Tronco Hematopoéticas , Humanos , Lactente , Masculino , Camundongos , Células NIH 3T3 , Pancitopenia/complicações , Pancitopenia/terapia , Mutação PuntualRESUMO
The aim of the study was to determine peculiarities of the distribution, diagnosis and development of immune cytopenias in patients with chronic lymphocytic leukemia (CLL) and to evaluate the efficacy of the different therapeutic approaches. MATERIALS AND METHODS: Treatment response and survival of 83 patients with CLL complicated by immune cytopenia (IC) were analyzed. Treatment schedules in 58 medicated patients included corticosteroids; chemotherapy (COP, CHOP regimens), immunotherapy (rituximab alone), immunochemotherapy (rituximab-containing regimens - R-COP, R-CHOP). Twenty-five patients underwent splenectomy. RESULTS: The use of corticosteroids, as the first line of treatment, resulted in short-term remission in most patients. Chemotherapy was effective in a half of CLL patients, but duration of the remission did not exceed 32 months in CLL associated with autoimmune hemolytic anemia and immune thrombocytopenia. After rituximab monotherapy (10 patients) the stable remission was reached in 60% of the patients with median relapse-free survival of 40 months. Rituximab containing chemotherapy (22 patients) caused the long-term remission in 72% of the patients with median relapse-free survival of 76 months. Splenectomy performed in 25 patients with CLL complicated by IC was effective in 70% of the patients. The outcome of splenectomy depends on IC entity. The best response was registered in associated immune thrombocytopenia (median overall survival 118 months), the worst - in Fisher - Evans syndrome (15 months). CONCLUSIONS: The treatment of patients with CLL complicated by ICs should be individualized. For CLL patients without significant enlargement of lymph nodes and spleen, low lymphocytosis, associated with autoimmune hemolytic anemia or immune thrombocytopenia, the monotherapy with rituximab is optimal. In case of occurrence of autoimmune hemolytic anemia, immune thrombocytopenia or Fisher - Evans syndrome in CLL patients with enlargement of lymph nodes, spleen, significant lymphocytosis, the use of R-COP or R-CHOP schemes, 4-6 courses, is the most effective. Splenectomy is indicated in patients with massive splenomegaly, the resistance to medication, recurrent relapses after adequate therapy.
Assuntos
Autoimunidade , Leucemia Linfocítica Crônica de Células B/complicações , Pancitopenia/diagnóstico , Pancitopenia/etiologia , Pancitopenia/terapia , Adulto , Idoso , Biomarcadores , Terapia Combinada , Gerenciamento Clínico , Suscetibilidade a Doenças , Feminino , Humanos , Imuno-Histoquímica , Imunofenotipagem , Leucemia Linfocítica Crônica de Células B/mortalidade , Masculino , Pessoa de Meia-Idade , Pancitopenia/mortalidade , Prognóstico , Modelos de Riscos Proporcionais , Resultado do TratamentoRESUMO
CD34+ immunomagnetic positive selection allows for CD34+ hematopoietic progenitors separation from CD3+ lymphocytes subsets, usually from an apheresis product collected from a previously mobilized donor. This T-cell depleted stem cell graft is primarily intended for rare cases (around 2% of allotransplanted patients in France) of severe, persistent, symptomatic bi- or tri-cytopenia post-allotransplantation, in order to allow for hematologic reconstitution without increasing the risk of GvHD occurrence. Although semi-manual and complex, the process is of sufficient robustness to consistently generate a cellular product with distinctive features and specifications, based on iterative in-process quality controls, that are discussed within these guidelines.
Assuntos
Antígenos CD34 , Células-Tronco Hematopoéticas/citologia , Separação Imunomagnética/métodos , Separação Imunomagnética/normas , Controle de Qualidade , Citaferese , Doença Enxerto-Hospedeiro/prevenção & controle , Células-Tronco Hematopoéticas/imunologia , Humanos , Pancitopenia/terapia , Sociedades MédicasRESUMO
A 35-year-old woman presented with a widespread petechial rash and pancytopenia. She underwent simultaneous pancreas and kidney transplantation for type 1 diabetes 8 years previously followed by a renal transplant 1 year prior to presentation, and was taking tacrolimus as long-term immunosuppression. The full blood count showed haemoglobin 97 g/L, platelet count 2×109/L and neutrophil count 0.22×109/L. Peripheral blood film examination confirmed genuine thrombocytopenia in the absence of any haemolytic or malignant features. Serological testing identified autoantibodies against all three blood lineages, consistent with a diagnosis of autoimmune pancytopenia. Treatment with steroids, intravenous immunoglobulins, romiplostim and mycophenolate mofetil achieved only fleeting remissions. Blood counts eventually normalised following the administration of rituximab and a change from tacrolimus to ciclosporin immunosuppression. Cytopenias are a well-recognised complication of post-transplantation care but we believe this to be the first reported case of autoimmune pancytopenia following solid organ transplantation. In this case report, we discuss the approach to investigation of haematological abnormalities post-transplant and the rationale for, and outcome of, the management of this rare case.
Assuntos
Doenças Autoimunes , Transplante de Rim , Transplante de Pâncreas , Pancitopenia/imunologia , Complicações Pós-Operatórias/imunologia , Adulto , Doenças Autoimunes/diagnóstico , Doenças Autoimunes/terapia , Feminino , Humanos , Pancitopenia/diagnóstico , Pancitopenia/terapia , Complicações Pós-Operatórias/diagnóstico , Complicações Pós-Operatórias/terapia , Fatores de TempoRESUMO
A 7-year-old boy presented with a constellation of bone pain, a skeletal lesion, and pancytopenia after undergoing allogeneic haematopoietic stem cell transplantation for recurrent acute B-cell lymphoblastic leukaemia. Investigations to rule out leukaemia recurrence were unremarkable. Due to presence of maturation arrest in erythropoiesis with giant pronormoblasts and aberrant intranuclear inclusions on a bone marrow aspirate, parvovirus B19 (PVB-19) staining was completed and confirmed the diagnosis of disseminated PVB-19. Though PVB-19 infection after solid organ transplantation was reported in the literature as early as 1986, acquired PVB-19 viremia presenting with a solitary bone lesion is a novel presentation in paediatrics.
Assuntos
Transplante de Células-Tronco Hematopoéticas , Infecções por Parvoviridae/diagnóstico , Parvovirus B19 Humano , Leucemia-Linfoma Linfoblástico de Células Precursoras/terapia , Biópsia , Criança , Terapia Combinada , Diagnóstico Diferencial , Humanos , Masculino , Dor/virologia , Pancitopenia/terapia , Pancitopenia/virologia , Infecções por Parvoviridae/patologia , Infecções por Parvoviridae/terapia , Leucemia-Linfoma Linfoblástico de Células Precursoras/diagnóstico , Recidiva , Viremia/diagnóstico , Viremia/patologia , Viremia/terapiaRESUMO
A 21-year-old man presented to the emergency department with generalised weakness, weight loss and decreased appetite for few weeks. He had evidence of severe pancytopenia and haemolysis. His peripheral smear with many schistocytes was suspicious for thrombotic thrombocytopenic purpura (TTP). He was supported with blood transfusions and daily plasmapheresis. His platelet counts worsened despite 4 days of therapy. Bone marrow biopsy was significant for hypercellular bone marrow with megaloblastic changes. Further workup revealed normal ADAMTS13 level, low vitamin B12, positive intrinsic factor antibodies and high methylmalonic acid. Diagnosis of pernicious anaemia was established and he was started on daily treatment with intramuscular vitamin B12 which subsequently improved his symptoms and haematological parameters. This report highlights the importance of checking vitamin B12 level in patients presenting with pancytopenia and TTP-like picture before making a diagnosis of TTP.
Assuntos
Anemia Perniciosa/complicações , Anemia Perniciosa/diagnóstico , Pancitopenia/diagnóstico , Pancitopenia/etiologia , Púrpura Trombocitopênica Trombótica/diagnóstico , Púrpura Trombocitopênica Trombótica/etiologia , Anemia Perniciosa/terapia , Humanos , Masculino , Pancitopenia/terapia , Púrpura Trombocitopênica Trombótica/terapia , Adulto JovemRESUMO
A 45-year-old man with idiopathic aplastic anemia required renal replacement therapy (RRT) due to end-stage renal disease (ESRD). We succeeded in inserting the peritoneal dialysis (PD) catheter under cover of frequent red blood cell and platelet infusions because of severe pancytopenia. During the one-year period after starting PD using an ultraviolet-ray sterilization device, he developed severe leukopenia but no PD-related peritonitis or exit site/tunnel infection until he died of pneumonia. This case suggests that PD might be a suitable choice as RRT in ESRD patients with aplastic anemia, even in those with severe pancytopenia.